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Desmoplakin I+II antibody

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  • ¥1380 - 2200
  • 康朗生物
  • 中国/美国/德国
  • 2025年12月01日
  • ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500
  • Rabbit
  • Human, Mouse, Rat, Cow, Rabbit, Sheep,
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    • 详细信息
    • 文献和实验
    • 技术资料
    • 供应商

      上海康朗生物科技有限公司

    • 库存

      大量

    • 目录编号

      kl-12953R

    • 克隆性

      多克隆

    • 抗原来源

      Rabbit

    • 保质期

      12个月

    • 抗体英文名

      Desmoplakin I+II antibody

    • 抗体名

      桥粒斑蛋白1+2抗体

    • 宿主

      Rabbit

    • 适应物种

      Human, Mouse, Rat, Cow, Rabbit, Sheep,

    • 免疫原

      KLH conjugated synthetic peptide derived from human DP-I:271-370/2871

    • 亚型

      IgG

    • 形态

      冻干粉或液体

    • 应用范围

      ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500

    • 浓度

      1mg/ml

    • 保存条件

      -20 °C

    • 规格

      100ul 200ul

    Desmoplakin I+II antibody
    中文名称 桥粒斑蛋白1+2抗体
    别    名 250/210 kDa paraneoplastic pemphigus antigen; Desmoplakin (DPI DPII); Desmoplakin; Desmoplakin I; Desmoplakin II; DESP_HUMAN; DP; DP I; DP II; DPI; DPII; DSP; KPPS2; PPKS 2; PPKS2.  
    规格价格 100ul/1380元 购买    200ul/2200元 购买    大包装/询价
    说 明 书 100ul  200ul
    研究领域 细胞生物  转录调节因子  细胞外基质  
    抗体来源 Rabbit
    克隆类型 Polyclonal
    交叉反应 Human, Mouse, Rat, Cow, Rabbit, Sheep, 
    产品应用 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 331kDa
    细胞定位 细胞浆 细胞膜 
    性    状 Lyophilized or Liquid
    浓    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human DP-I:271-370/2871 
    亚    型 IgG
    纯化方法 affinity purified by Protein A
    储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    PubMed PubMed
    产品介绍 background:
    Desmosomes are intercellular junctions that form tight links between adjacent cells. Desmoplakin is an obligate component of functional desmosomes that attaches intermediate filaments to desmosomal plaques. It is involved in the organization of desmosomal cadherin-plakoglobin complexes into discrete plasma membrane domains. The N-terminus of desmoplakin is essential for localisation to the desmosome and interaction with plakophilin 1 and plakoglobin. The C-terminus of desmoplakin binds to intermediate filaments. The central region of desmoplakin comprises a coiled-coil rod domain that mediates homodimerisation. There are two isoforms of desmoplakin - desmoplakin I, which is an obligate component of all desmosomes, and desmoplakin II, which is predominantly expressed in tissues and cells of stratified origin. Mutations in the gene encoding desmoplakin result in a number of cardiomyopathies and keratodermas as well as the autoimmune disease paraneoplastic pemphigus.

    Function:
    Major high molecular weight protein of desmosomes. Involved in the organization of the desmosomal cadherin-plakoglobin complexes into discrete plasma membrane domains and in the anchoring of intermediate filaments to the desmosomes.

    Subunit:
    Homodimer. Interacts with COL17A1 (via cytoplasmic region). Associates (via C-terminal) with KRT5-KRT14 (via rod region), KRT8-KRT18 and VIM intermediate filaments. Interacts with DSC2.

    Subcellular Location:
    Cell junction, desmosome. Cytoplasm, cytoskeleton. Note=Innermost portion of the desmosomal plaque. Colocalizes with epidermal KRT5-KRT14 and simple KRT8-KRT18 keratins and VIM intermediate filaments network.

    Tissue Specificity:
    Isoform DPI is apparently an obligate constituent of all desmosomes. Isoform DPII resides predominantly in tissues and cells of stratified origin.

    Post-translational modifications:
    Ser-2849 is probably phosphorylated by a cAMP-dependent protein kinase. Phosphorylation on Ser-2849 probably affects its association with epidermal, simple cytokeratins and VIM intermediate filaments.
    Substrate of transglutaminase. Some glutamines and lysines are cross-linked to other desmoplakin molecules, to other proteins such as keratin, envoplakin, periplakin and involucrin, and to lipids like omega-hydroxyceramide.

    DISEASE:
    Defects in DSP are the cause of palmoplantar keratoderma striate type 2 (SPPK2) [MIM:612908]; also known as keratosis palmoplantaris striata II. SPPK2 is characterized by skin thickening in the palms (linear pattern) and the soles (island-like pattern) and flexor aspect of the fingers. Abnormalities of the nails, the teeth and the hair are rarely present. 
    Defects in DSP are the cause of cardiomyopathy dilated with woolly hair and keratoderma (DCWHK) [MIM:605676]; also known as Carvajal syndrome or palmoplantar keratoderma with left ventricular cardiomyopathy and woolly hair. DCWHK is an autosomal recessive cardiocutaneous syndrome characterized by a generalized striate keratoderma particularly affecting the palmoplantar epidermis, woolly hair, and 
    Defects in DSP are the cause of familial arrhythmogenic right ventricular dysplasia type 8 (ARVD8) [MIM:607450]; also known as arrhythmogenic right ventricular cardiomyopathy 8 (ARVC8). ARVD is an autosomal dominant disease characterized by partial degeneration of the myocardium of the right ventricle, electrical instability, and sudden death. It is clinically defined by electrocardiographic and angiographic criteria; pathologic findings, replacement of ventricular myocardium with fatty and fibrous elements, preferentially involve the right ventricular free wall. 
    Defects in DSP are the cause of skin fragility-woolly hair syndrome (SFWHS) [MIM:607655]. SFWHS is an autosomal recessive genodermatosis characterized by focal and diffuse palmoplantar keratoderma, hyperkeratotic plaques on the trunk and limbs, and woolly hair with varying degrees of alopecia. 
    Defects in DSP are the cause of epidermolysis bullosa lethal acantholytic (EBLA) [MIM:609638]. EBLA is characterized by severe fragility of skin and mucous membranes. The phenotype is lethal in the neonatal period because of immense transcutaneous fluid loss. Typical features include universal alopecia, neonatal teeth, and nail loss. Histopathology of the skin shows suprabasal clefting and acantholysis throughout the spinous layer, mimicking pemphigus.

    Similarity:
    Belongs to the plakin or cytolinker family.
    Contains 17 plectin repeats.
    Contains 1 SH3 domain.
    Contains 6 spectrin repeats.

    SWISS:
    P15924

    Gene ID:
    1832

    Database links:

    Entrez Gene: 420869 Chicken

    Entrez Gene: 514360 Cow

    Entrez Gene: 1832 Human

    Entrez Gene: 109620 Mouse

    Entrez Gene: 306871 Rat

    Omim: 125647 Human

    SwissProt: P15924 Human

    SwissProt: E9Q557 Mouse

    Unigene: 519873 Human

    Unigene: 355327 Mouse



    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

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    图标文献和实验
    相关实验
    • Protein A Purification of Antibody

      1. Reagents(1) Affi-gel Protein-A Agarose (BioRad #153-6153)(2) MAPS II Binding Buffer (BioRad # 153-6161)(3) 0.314 g/ml diH2O(4) MAPS II Elution Buffer (BioRad #153-6162)(5) 0.023 g/ml diH2O(6) MAPS II Regeneration Buffer (BioRad #153-6166(7) 1M

    • Identification of MHC Class II Binding Peptides: Microarray and Soluble MHC Class II Molecules

      antibody (mAb). Positive events (MHC class II/peptide complexes) are normalized and available for pattern analysis.

    • 大鼠MHC II(MHC II)ELISA试剂盒 说明书

      上海西唐生物科技有限公司 021-55229872, 65333639 www.westang.com 大鼠 MHC II(MHC II)ELISA 试剂盒 ( 用于血清、血浆、细胞培养上清液和其它生物体液内 ) 原理 本实验采用双抗体夹心 ABC-ELISA 法。用抗大鼠 MHC II 单抗包被于酶标板上,标准品和样品中的 MHC II 与单抗结合,加入生物素化的抗大鼠 MHC II ,形成免疫复合

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